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Malignant Tumours
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PaoloBattaglia, GiorgioSileo,
andPaoloCastelnuovo
32
Introduction
Sinonasal malignancies are rare tumours,
accounting for 0.2–0.8% of all malignancies and
3–5% of head and neck cancers. Their prognosis
is extremely variable, being inuenced by the
local extension of the disease, possible involvement of noble structures such as brain, orbit or
internal carotid artery and tumour histology,
itself critically inuencing the biological aggressiveness [1].
The management of these uncommon diseases
is handled by a multidisciplinary oncologic skull
base team composed of head and neck surgeons,
neurosurgeons, radiologists, radiotherapists,
medical oncologists and pathologists.
P. Battaglia (*) · P. Castelnuovo
Division of Otorhinolaryngology, Department of
Biotechnology and Life Sciences, , University of
Insubria-Varese, ASST Sette Laghi, Ospedale di
Circolo e Fondazione Macchi, Varese, Italy
Department of Biotechnology and Life Sciences,
Head and Neck Surgery and Forensic Dissection
Research Center (HNS and FDRc), University of
Insubria, Varese, Italy
G. Sileo
Department of Biotechnology and Life Sciences,
Head and Neck Surgery and Forensic Dissection
Research Center (HNS and FDRc), University of
Insubria, Varese, Italy
The spectrum of treatment strategies is wide,
from various surgical approaches to multimodal
management, and is driven by the tumour histotype and its extension.
Craniofacial resection, rstly described by
Ketcham in 1964 [2], has represented the gold
standard in the treatment of sinonasal malignant
tumours for decades, even though it is burdened
by invasive transfacial approaches, signicant
functional sequelae and a complication and mortality rate of 36.3% and 4.7%, respectively [3].
The endoscopic endonasal approach, which
was initially developed in the 1970s for the treatment of inammatory sinonasal conditions, following progressive renements in surgical
techniques and technologies, has been gradually
© The Author(s), under exclusive license to Springer Nature Switzerland AG 2023
A. C. Swift et al. (eds.), Contemporary Rhinology: Science and Practice,
https://doi.org/10.1007/978-3-031-28690-2_32
415
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P. Battaglia et al.
applied to selected cases of sinonasal malignancies since 1990 [4], with results comparable to
those ones of traditional external approaches.
Epidemiology
In Western countries the incidence varies between
0.8 and 1 per 100,000 people, whereas it is greater
in Africa and Eastern countries, where it can reach
2.6 per 100,000 people as reported in Japan [5].
The average age at diagnosis is 60years; men
are more affected than females (58.6%), which is
probably due to environmental or occupational
exposure. Children can be affected by different
histotypes, rhabdomyosarcoma being the most
prevalent type [6].
The most common site of origin is the maxil-
lary sinus (50–70%), followed by the nasal cavity
(15–30%) and ethmoid sinus (10–20%); frontal
and sphenoid sinuses are rarely the primary site,
yet they are generally involved by locally
advanced tumours with dismal prognosis.
The role of different work-related chemical
hazards in determining sinonasal cancers has
been widely investigated by epidemiological
studies, and the evaluation of the occupational
exposure can be very challenging, because of the
potential long latency period.
Workers exposed to wood dust, leather, alumin-
ium and other chemicals (such as formaldehyde
and solvents) have an increased risk for developing sinonasal malignancies. The strong association
between intestinal-type adenocarcinoma (ITAC)
and former exposure to wood or leather dust, as
demonstrated by Bonzini et al. (87% of patients
with ITAC were exposed), is noteworthy [7].
In addition to occupational hazards, other risk
factors include previous head and neck irradiation, smoking, genetic alterations and inverted
papilloma.
Two thirds of sinonasal malignancies have an
epithelial origin and the most common histologies
are adenocarcinoma (ADC) in European countries and squamous cell carcinoma (SCC) in North
America; other epithelial histotypes include adenoid cystic carcinoma (ACC) and sinonasal undifferentiated carcinoma (SNUC) [8].
Paranasal sinuses may be the site of metastases from other cancers and almost half of all
cases is represented by renal cellular carcinoma,
followed by breast, prostate, lungs, gastrointestinal tract and thyroid carcinoma [9].
Clinical Features
Sinonasal malignancies commonly present with
non-specic signs and symptoms, therefore making the diagnosis difcult and generally delayed.
Initial ndings can often be misleading because
they may mimic more common and benign conditions such as inammatory diseases.
In our institution, among 565 patients treated
in the last 20years, the most common complaints
were respiratory nasal obstruction (71%), epistaxis (51%), olfactory dysfunction (36%), rhinorrhoea (29%), headache (17%), facial pain
(13%), epiphora (6%), swelling (4%), visual disturbance (4%) and diplopia (4%).
Symptoms may be an indicator of the local
extension of the disease because of the mass
effect on surrounding tissues.
Maxillary tumours may present with facial
swelling, if extending anteriorly, or palatal
swelling and loosening of teeth, in cases with
inferior extension; diplopia and proptosis may
be the result of orbital invasion, whereas a posterior spread, towards the infratemporal/pterygoid palatine fossa, may cause trismus or facial
neuralgia or occasionally altered sensation/
numbness because of the involvement of masticatory muscles or maxillary nerve. Ethmoidal
malignancies may extend laterally into the orbit,
thus causing visual symptoms or proptosis, or
intracranially with potential neurological symptoms (Fig.32.1).
Among this broad spectrum of clinical ndings, unilateral persistent symptoms unresponsive to medical treatment must draw clinicians’
attention and should prompt a thorough further
investigation.
On initial presentation cervical lymph node
metastases occur within a range variable from 3
to 30%, whereas distant metastases are less frequent, with an incidence of 1–7% [10].
bc
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a
417
Fig. 32.1 Potential directions of growth of ethmoidal
(yellow star) and maxillary (red star) tumours in different
CT scan views ((a), coronal; (b) axial at the level of maxillary sinuses; (c) axial at the level of the orbits). Legend:
1 orbital inltration; 2 nasal cavity extension; 3 ante rior/
Diagnostic Workup
In cases of suspected sinonasal expansile
lesions, the patient must be referred to an
otolaryngologist for a complete clinical
examination.
Nasal endoscopy, performed with exible or
rigid scopes, is the rst diagnostic test of utmost
importance since it can detect the lesion, its characteristics (e.g. ulceration, bleeding) and its possible site of origin.
CT Imaging
The second step is computed tomography (CT)
imaging, generally done without contrast, to
evaluate the sinonasal anatomy and the presence
of bony alterations, which can present with different patterns:
• Bone remodelling, that is displacement and
thinning of bony structures (more frequently
observed in benign neoplasms or chronic
inammatory processes)
• Cortical destruction, that is interruption in the
whole thickness of mineralized bone
• Intra-diploic growth, in cases of intra-osseous
spread, that is the replacement of spongiosa
by solid tissue
• Permeative invasion, that is subperiosteal
spread with diffuse demineralization (mostly
lateral maxillary wall inltration; 4 contralateral nasal
cavity extension; 5 intracranial invasion; 6 exten sion into
the oral cavity; 7 posterior extension into the pterygopalatine or infratemporal fossa
observed in lymphomas and adenoid cystic
carcinomas)
• Sclerosis, as a result of chronic inammatory
reaction of the spongiosa [11]
The CT scan facilitates the evaluation of the
lamina papyracea and skull base, thus providing
preliminary details of intraorbital and intracranial
extension. Moreover, the enlargement of bony ssures or foramina may be an indicator of perineural
spread. Lastly, modern CT scans with three-dimensional reconstruction in axial, coronal and sagittal
planes are crucial in surgical planning.
MRI Imaging
The third step consists of magnetic resonance
imaging (MRI) scan with contrast (gadolinium),
which has the potential to differentiate soft tissue
densities and to assess the grade of vascularization. MRI of the head is strongly recommended
on occasions where a CT head scan demonstrates
unexpected unilateral sinonasal mass in patients
with no sinonasal symptoms.
A systematic approach to different MRI
sequences is crucial in characterizing the lesion
and in evaluating its relationship with adjacent
structures. For this purpose, it is useful to compare T2 with plain T1 and contrast-enhanced T1
sequences: The rst shows uid as bright, the second highlights fat as bright, whereas the latter
enhances vascularized neoformations, which are
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P. Battaglia et al.
usually hypointense on T2. “Fat-sat” (applicable
to both T1 and T2) is another useful sequence that
suppresses fat signal, hence helping in delineating
the relationship between tumour and fat tissue.
The usefulness of MRI is demonstrated by its
ability to assess eventual inltration of the orbit,
anterior cranial fossa and pterygopalatine/infratemporal fossa, which dramatically inuences
the treatment planning.
Orbital walls appear hypointense on T1 and
T2 sequences because of the reduced water content of lamina papyracea and periorbita; thus,
neoplastic inltration is suspected when the
hypointense interface is not recognizable.
T2 and contrast-enhanced T1 sequences
enable evaluation of different stages of anterior
cranial fossa invasion, by looking at its three different layers (cribriform plate, dura and cerebrospinal uid); indeed, a thickened and enhanced
dura suggests skull base invasion.
Posterior extension to the pterygopalatine and
infratemporal fossa is demonstrated by maxillary
bone erosion, loss of fat signal or altered signal
intensity of the pterygoid muscles [12].
However, it is important to appreciate that in
almost all cases CT and MRI ndings are nonspecic and do not allow to differentiate between
different malignant histotypes.
As a general rule, biopsy is best performed
after completing imaging studies to minimize the
risk of bleeding from vascular tumours (e.g. juvenile angiobroma, meningoencephalocele).
Biopsy is generally performed under local anaesthesia with rigid scopes, but in some cases, general anaesthesia is required.
A pathological second opinion in centres with
dedicated expertise is strongly recommended in
order to conrm the denitive diagnosis and plan
the adequate treatment.
Additional Scanning Protocols
Once a malignant tumour has been conrmed, it
is essential to exclude or identify regional and/or
distant metastatic disease, according to the policy
of the local radiology department. This typically
includes ultrasound neck and CT chest and
abdomen.
Total body positron emission tomography
(PET-CT) scan is preferred in cases of aggressive
histotypes (e.g. mucosal melanoma, neuroendocrine carcinoma) or advanced stages.
Staging
Different staging systems have been developed in
the past decades to evaluate sinonasal
malignancies.
The Union for International Cancer Control/
American Joint Committee on Cancer (UICC/
AJCC) TNM classication, now in its eighth edition, is the most widely used. In this staging system, the T classication depends on the progressive
involvement of adjacent structures; the sinonasal
tract is divided into maxillary sinus and nasal cavity/ethmoid sinus. All histotypes are included
except for mucosal melanoma, which has its own
TNM classication (T3 is the minimum) due to its
extremely aggressive behaviour [13].
Different staging systems have been proposed
for esthesioneuroblastoma, because of its peculiar biological behaviour: The Kadish classication, which was developed in 1976, is the most
commonly used and divides patients into three
categories [14]; a fourth new category, for
patients with metastases, was introduced by
Morita in 1993. In 1992 Dulguerov and
Calcaterra developed a new staging system,
which was found to be better correlated with survival [15].
The Wang staging system was developed for
carcinoma of the nasal vestibule, which is an
aggressive cancer with a worse prognosis than
other head and neck skin cancers; this staging
system is based on the invasion depth and is a
better prognostic indicator than the TNM classication [16] (Tables 32.1, 32.2, 32.3, 32.4 and
32.5).
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Table 32.1 T staging according to the AJCC eighth edition
Maxillary sinus Nasal cavity and ethmoid sinus
T1 Tumour limited to the mucosa (no
erosion or destruction of the bone)
T2 Tumour causing bone erosion or
destruction (hard palate and/or middle
meatus extension is included, whereas
extension to posterior maxillary wall
and pterygoid plates is excluded)
T3 Tumour involves any of the following:
• Posterior maxillary bony wall
• Floor or medial orbital wall
• Subcutaneous tissues
• Pterygoid fossa
• Ethmoid sinuses
T4a Tumour involves any of the following:
• Anterior orbital contents
• Skin of the cheek
• Pterygoid plates
• Sphenoid or frontal sinuses
• Cribriform plate
• Infratemporal fossa
T4b Tumour involves any of the following:
• Orbital apex
• Dura
• Brain
• Middle cranial fossa
• Cranial nerves other than V2
• Nasopharynx or clivus
a
Anatomical site and subsites: nasal cavity (septum, oor, lateral wall, vestibule), maxillary sinus, ethmoid sinus (left, right)
Tumour restricted to one subsitea of
the nasal cavity or ethmoid sinus
Tumour involves two subsites in a
single site or involves an adjacent
sites within the nasoethmoidal
complex
Tumour involves any of the
following:
• Maxillary sinus
• Floor or medial orbital wall
• Palate
• Cribriform plate
Tumour involves any of the
following:
• Anterior orbital contents
• Skin of the nose or cheek
• Pterygoid plates
• Sphenoid or frontal sinuses
• Minimal extension to anterior
cranial fossa
Tumour involves any of the
following:
• Orbital apex
• Dura
• Brain
• Middle cranial fossa
• Cranial nerves other than V2
• Nasopharynx or clivus
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Table 32.2 N staging according to the AJCC eighth edition
Regional lymph nodes
Nx Regional lymph nodes cannot be assessed
N0 No regional lymph nodes metastases
N1
N2a Metastasis in a single ipsilateral lymph node, between 3 and 6cm in greatest dimension (no extranodal
N2b
N2c
N3a Metastasis in a lymph node >6cm in greatest dimension (no extranodal extension)
N3b Metastasis in a single (>3cm) or multiple lymph nodes with extranodal extension
Metastasis in a single ipsilateral lymph node, ≤3cm in greatest dimension (no extranodal extension)
extension) or<3cm with extranodal extension
Metastasis in multiple ipsilateral lymph nodes, ≤6cm in greatest dimension (no extranodal extension)
Metastasis in bilateral or contralateral lymph nodes, ≤6cm in greatest dimension (no extranodal
extension)
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Table 32.3 T staging of malignant melanoma of upper airways and digestive tract according to AJCC eighth
edition
Malignant melanoma of upper airways and digestive tract
T3 Tumour is limited to the epithelium and/or submucosa
T4a Tumour involves the bone, cartilage, deep soft tissue or overlying skin
T4b Tumour involves any of the following:
• Brain
• Dura
• Skull base
• Lower cranial nerves (IX, X, XI, XII)
• Masticator space
• Carotid artery
• Prevertebral space
• Mediastinal structures
Table 32.4 Kadish-Morita and Dulguerov-Calcaterra staging system for esthesioneuroblastoma
Kadish-Morita Dulguerov-Calcaterra
A Tumour is limited to the nasal
cavity
B Tumour involves the nasal
cavity and paranasal sinuses
C Tumour extends beyond the
nasal cavity and paranasal
sinuses
D Regional or distant metastases T4 Tumour involves the brain
T1 Tumour involves the nasal cavity and/or
paranasal sinuses (excluding sphenoid), sparing
the most superior ethmoidal cells
T2 Tumour involves the nasal cavity and/or
paranasal sinuses (including the sphenoid), with
extension to or erosion of cribriform plate
T3 Tumour extends into the orbit or protrudes into
the anterior cranial fossa, without dura invasion
P. Battaglia et al.
Table 32.5 Wang T staging system for carcinoma of the
nasal vestibule
Wang T staging system
T1 Tumour conned to the skin
T2 Tumour invades subcutaneous tissue and
cartilage
T3 Tumour invades the bone
Histology-Driven Treatments
In this section the most common histotypes and
their multimodal treatment protocols are
presented.
Squamous Cell Carcinoma (SCC)
SCC is the most common sinonasal malignancy in the United States: It originates in the
maxillary sinus in 60% of cases, less frequently
in the nasal cavity or ethmoid. Tumours occur
in men twice as much as in women in their 50s
and 60s.
It can present with different subtypes: keratinizing (KSCC), non-keratinizing (NKSCC) and
other rarer variants.
KSCC is the most common (50% of cases)
and is characterized by keratinization; indeed
epithelial markers (e.g. citokeratins) are
expressed. It is identical to KSCC arising in other
sites and it can be found in approximately 5–10%
of sinonasal inverted papillomas [17].
NKSCC accounts for 20% of sinonasal SCC
and is similar to that one arising in the oropharynx.
It is characterized by minimal squamous differentiation and does not have tumour grading. The
association with high-risk HPV is found in almost
50% of cases and correlates with a trend towards
improved survival [18].
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The standard treatment is surgical resection
followed by adjuvant radiotherapy; irradiation
should include the neck in case of advanced
stages (T3–T4), given the high risk of nodal
metastases (20%). In case of positive margins or
evidence of neural/lymphovascular inltration,
adjuvant chemotherapy can be administered.
Patients with high-grade carcinoma in
advanced stages (T3–T4) can be treated with
induction chemotherapy regimens followed by
surgery and postoperative chemoradiation or
denitive chemoradiation; tumour response to
induction chemotherapy is associated with better
survival and prognosis [19] (Fig.32.2).
a
de f
Fig. 32.2 A 54-year-old female affected by right maxillary sinus squamous cell carcinoma G2 extending into the
infratemporal fossa, parotid, temporalis muscle and subcutaneous cheek (T4aN0M0). Preoperative CT (a) and
MRI scans ((b) T2W; (c) T1W with contrast) in coronal
views. The patient underwent craniofacial resection with
right selective neck dissection (I–IV), reconstruction with
anterolateral thigh free ap and adjuvant radiotherapy
(60Gy). MRI scans in coronal views ((d) T2W; (e) T1W
with contrast) and nasal endoscopy (f) at 1-year follow up: the patient is alive without disease. Legend: Alt
anterolateral thigh free ap, Ion infraorbital nerve, Np
nasopharynx, Ns nasal septum, Ss sphenoid sinus, T
tumour, black arrows erosion of the lateral maxillary wall
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Adenocarcinoma (ADC)
ADC is the most common sinonasal epithelial
malignancy in Europe and it generally originates
in the ethmoid (85%), followed by the olfactory
cleft (13%).
Intestinal-type adenocarcinoma (ITAC) takes
its name from the morphological analogy with
intestinal adenocarcinomas and correlates with
occupational exposure to leather or wood dust in
up to 87% of cases [7]. It affects predominantly
men aged between 40 and 70years.
According to Barnes’s classication, different
subtypes can be distinguished: papillary (75% of
cases), solid, mucinous (e.g. signet-ring cells)
and mixed. Solid and mucinous patterns are
indicative of poorly differentiated cancers, thus
behaving more aggressively [20].
a
Non-intestinal-type adenocarcinoma (nITAC)
represents a diagnosis of exclusion; features of
intestinal or salivary gland tumours are absent;
positive for markers of seromucinous differentiation (e.g. S100, DOG1) are often demonstrated.
As opposed to ITAC, it is not correlated with
occupational exposure and patients are generally
younger (50s) with a mild female predilection.
Surgical resection is the mainstay of treatment:
It is the single effective treatment for low- grade
tumours in early stages (T1–T2), but it should be
followed by postoperative radiotherapy (PORT)
in case of high-grade neoplasms, advanced stages
(T3–T4) or inltrated surgical margins. In case of
high-grade lesions with intracranial extension, a
prophylactic brain irradiation should also be considered, given the potential risk of leptomeningeal
involvement [21] (Fig. 32.3).
def
Fig. 32.3 A 78-year-old male, former woodworker,
affected by right sinonasal intestinal-type adenocarcinoma G1 (T3N0M0). Preoperative MRI scans in coronal
views ((a) T2W; (b) T1W with contrast). Right endoscopic resection with transnasal craniectomy (ERTC) and
skull base reconstruction with fascia lata and septal ip
ap was performed ((c) right nasal fossa intraoperative
view after dura removal), adjuvant radiotherapy followed
(66Gy). MRI scans in coronal views ((d) T2W; (e) T1W
with contrast) and nasal endoscopy (f) after 5years demonstrate no evidence of the disease. Legend: B brain, D
dura mater, Fs frontal sinus, Lp lamina papyracea, Ns
nasal septum, Sff and white arrowheads septal ip ap, Ss
sphenoid sinus, T tumour
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The neck is not routinely treated as regional
metastases occur in only 7% of patients.
Induction chemotherapy has been proposed for
advanced- stage (T3–T4) ITAC with functional
p53, showing promising results in survival
[22].
Adenoid Cystic Carcinoma (ACC)
ACC is a rare salivary gland tumour that involves
most frequently the maxillary sinus (60%) and
the nasal cavity (30%). It has a slight prevalence
in women, with a peak of incidence in the fth
and sixth decades.
Given the strong propensity for perineural
and bony spread, intracranial extension (including cavernous sinus) is likely and local recurrence is common (60%). Another important
characteristic is distant metastases (lung, bone
and brain), often presenting many years after the
initial tumour and occurring in approximately
40% of patients [23].
ACC presents in different subtypes: cribriform, tubular and the less differentiated solid.
Different grading systems have been proposed to
emphasize the importance of histological subtypes; indeed, according to the Perzin/Szanto
system, ACC is classied as high grade if the
solid component represents more than 30%. In
this case the tumour behaves locally more
aggressively and tends to develop early distant
metastases [24].
ACC is considered a relative radiosensitive
tumour; hence the standard treatment is surgical
radical resection, whenever feasible, followed by
adjuvant radiotherapy to clear positive margins
(microscopic or macroscopic) [25].
In cases of locally advanced stages, with
involvement of vital structures, function-sparing
tumour debulking reduces the target volume,
making PORT more selective and efcient.
Heavy particle radiotherapy, with protons or
carbon ions, has recently been introduced and
demonstrates improved local control, both for
postoperative patients and those with unresectable ACC. A signicant advantage of this technique is the ability to deliver high tumouricidal
doses whilst sparing adjacent normal tissues.
Esthesioneuroblastoma (ENB)
ENB, also named olfactory neuroblastoma, is a
malignant neuroectodermal neoplasm that arises
from the olfactory neuro-epithelium. It has a
slight predominance in male (male-to-female
ratio 1.2:1), and although a bimodal distribution
in age has been initially reported, it affects
patients in the fth or sixth decade [26].
It is typically located in the superior portion of
the nasal vault and involves the cribriform plate.
Ectopic location within the paranasal sinuses is
extremely rare. It can present with a paraneoplastic syndrome but only in 2% of patients (e.g. syndrome of inappropriate antidiuretic hormone/
ADH secretion) [27].
Metastases at presentation are rare; furthermore they develop late in the natural history of
the disease, most frequently in cervical lymph
nodes. Several staging systems have been proposed, but the Kadish staging system is the most
commonly applied.
The Hyams grading system classies ENB
into four grades, from most (grade I) to least differentiated (grade IV), depending on specic histopathological features. Higher grades are
associated with more aggressive locoregional
disease and worse disease-free survival.
The differential diagnosis is wide, and immunohistochemistry is of utmost importance:
neuron- specic enolase, synaptophysin and
chromogranin A are typically positive. Review
of pathological specimens by expert pathologists
is crucial especially when dealing with poorly
differentiated ENBs, given that they could easily
be confused with other neuroendocrine tumours.
The standard treatment is surgical resection,
with removal of the anterior skull base dura and
olfactory bulb, followed by adjuvant radiotherapy; irradiation should include the neck in cases
of intracranial extension (Kadish C).
The role of chemotherapy is debated; however
neoadjuvant regimens (e.g. etoposide/cisplatin)
are generally advocated for patients with poorly
differentiated ENB in locally advanced stages
[28]. Follow-up should be long term, and should
metastatic neck disease present at a late stage,
neck dissection with possible PORT should be
considered (Fig.32.4).
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Fig. 32.4 A 58-year-old male affected by right esthesioneuroblastoma Kadish C, Hyams II. The preoperative
MRI scans in coronal views ((a) T1W with contrast; (b)
T2W) and intraoperative view (c) show the intracranial
extension of the disease. The patient underwent bilateral
ERTC and skull base reconstruction with fascia lata followed by adjuvant radiotherapy (60/54Gy on T/N). MRI
Neuroendocrine Carcinoma (NEC)
Sinonasal tumours with neuroendocrine differentiation is a heterogeneous group of rare neoplasms with neuroectodermal (ENB) or epithelial
origin (NEC).
NEC is a high-grade carcinoma with features
of neuroendocrine differentiation that accounts
for 5% of sinonasal malignancies; it is an aggressive tumour characterized by a dismal prognosis
with a high rate of local recurrences and distant
metastases (lung, liver and bones). It can be categorized into typical and atypical carcinoids and
small cell and large cell NECs.
It has a slight male predominance and a
median age at diagnosis of 56 years. The most
common site of origin is the ethmoid (64%), followed by the nasal cavity (32%) and the maxillary sinus (14%) [29].
scans in coronal views ((d) T1W with contrast; (e) T2W)
and nasal endoscopy (f) performed at 7-month follow-up
demonstrate local control of the disease. Legend: B brain,
D dura mater, D3 Draf III, Lp lamina papyracea, Sbr and
white arrowheads skull base reconstruction, Ti intradural
tumour, Te extradural tumour
NECs are strongly positive for cytokeratins,
epithelial membrane antigen and markers of neuroendocrine differentiation (e.g. synaptophysin);
according to a European multicentre study,
CK8/18 immunohistochemistry is strongly recommended in order to avoid a misdiagnosis of
ENB, due to the negative staining for CKAE1/A3
[28].
Mixed neuroendocrine-nonneuroendocrine
neoplasm is a recently described histopathological entity, in which the neuroendocrine component represents at least 30% of the lesion,
characterized by an aggressive biological behaviour with frequent recurrences (80%) and poor
survival outcomes [30].
The role of neoadjuvant chemotherapy is
still debated but promising, due to the frequent
distant failures and the chemosensitivity of
NEC; the rate of response to induction chemo-
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